{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T21:54:29Z","timestamp":1793397269554,"version":"4.5.0"},"reference-count":117,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T00:00:00Z","timestamp":1619827200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Pharmacological Research"],"published-print":{"date-parts":[[2021,5]]},"DOI":"10.1016\/j.phrs.2021.105593","type":"journal-article","created":{"date-parts":[[2021,4,4]],"date-time":"2021-04-04T09:37:32Z","timestamp":1617529052000},"page":"105593","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":37,"special_numbering":"C","title":["Beh\u00e7et disease: From pathogenesis to novel therapeutic options"],"prefix":"10.1016","volume":"167","author":[{"given":"Javier","family":"Rodr\u00edguez-Carrio","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Nucera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignazio Francesco","family":"Masala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabiola","family":"Atzeni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.phrs.2021.105593_bib1","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1056\/NEJM199910213411707","article-title":"Beh\u00e7et\u2019s disease","volume":"341","author":"Sakane","year":"1999","journal-title":"New Engl. J. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib2","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1007\/s11926-010-0132-z","article-title":"Beh\u00e7et\u2019s syndrome","volume":"12","author":"Yazici","year":"2010","journal-title":"Curr. Rheumatol. Rep."},{"key":"10.1016\/j.phrs.2021.105593_bib3","doi-asserted-by":"crossref","first-page":"81","DOI":"10.2174\/1568010053622894","article-title":"Beh\u00e7ets disease as an autoinflammatory disorder","volume":"4","author":"Gul","year":"2005","journal-title":"Curr. Drug Target -Inflamm. Allergy"},{"issue":"4","key":"10.1016\/j.phrs.2021.105593_bib4","first-page":"S1","article-title":"Beh\u00e7et\u2019s disease and other autoinflammatory conditions: what\u2019s in a name?","volume":"23","author":"Yazici","year":"2005","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib5","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.1136\/ard.2003.018515","article-title":"Cerebral venous thrombosis is associated with major vessel disease in Behcet\u2019s syndrome","volume":"63","author":"Tunc","year":"2004","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib6","first-page":"649","article-title":"Vascular involvements in Beh\u00e7et disease.\u00a0. Based on 40 patient records","volume":"74","author":"Kabbaj","year":"1993","journal-title":"J. Radiol."},{"key":"10.1016\/j.phrs.2021.105593_bib7","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1097\/BOR.0b013e32831de4e7","article-title":"Thromboembolic disease in vasculitis","volume":"21","author":"Tomasson","year":"2009","journal-title":"Curr. Opin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib8","doi-asserted-by":"crossref","first-page":"W158","DOI":"10.2214\/AJR.09.2763","article-title":"Pulmonary and vascular manifestations of beh\u00e7et disease: imaging findings","volume":"194","author":"Ceylan","year":"2010","journal-title":"Am. J. Roentgenol."},{"key":"10.1016\/j.phrs.2021.105593_bib9","article-title":"Vascular involvement in Behcet\u2019s disease","author":"Koc","year":"1992","journal-title":"J. Rheuma"},{"key":"10.1016\/j.phrs.2021.105593_bib10","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1038\/nrrheum.2017.208","article-title":"Beh\u00e7et syndrome: a contemporary view","volume":"14","author":"Yazici","year":"2018","journal-title":"Nat. Rev. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib11","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1093\/rheumatology\/kel329","article-title":"Autoimmunity vs autoinflammation in Behcet\u2019s disease: do we oversimplify a complex disorder?","volume":"45","author":"Direskeneli","year":"2006","journal-title":"Rheumatology"},{"key":"10.1016\/j.phrs.2021.105593_bib12","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.autrev.2009.10.005","article-title":"Etiopathogenesis of Behcet\u2019s disease","volume":"9","author":"Mendoza-Pinto","year":"2010","journal-title":"Autoimmun. Rev."},{"key":"10.1016\/j.phrs.2021.105593_bib13","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1038\/nrrheum.2015.147","article-title":"\u2019MHC-I-opathy\u2019\u2014unified concept for spondyloarthritis and Beh\u00e7et disease","volume":"11","author":"McGonagle","year":"2015","journal-title":"Nat. Rev. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib14","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1016\/j.autrev.2017.12.006","article-title":"Beh\u00e7et\u2019s disease: new insights into pathophysiology, clinical features and treatment options","volume":"17","author":"Greco","year":"2018","journal-title":"Autoimmun. Rev."},{"key":"10.1016\/j.phrs.2021.105593_bib15","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s10238-014-0328-z","article-title":"Beh\u00e7et syndrome: from pathogenesis to novel therapies","volume":"16","author":"Mazzoccoli","year":"2016","journal-title":"Clin. Exp. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib16","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/j.berh.2004.02.008","article-title":"Beh\u00e7et\u2019s disease","volume":"18","author":"Marshall","year":"2004","journal-title":"Best. Pract. Res. Clin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib17","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1097\/BOR.0000000000000189","article-title":"Endoplasmic reticulum-associated amino-peptidase 1 and rheumatic disease","volume":"27","author":"Ombrello","year":"2015","journal-title":"Curr. Opin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib18","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1038\/ng.2520","article-title":"Genome-wide association analysis identifies new susceptibility loci for Beh\u00e7et\u2019s disease and epistasis between HLA-B*51 and ERAP1","volume":"45","author":"Kirino","year":"2013","journal-title":"Nat. Genet."},{"key":"10.1016\/j.phrs.2021.105593_bib19","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0161305","article-title":"Genetic analysis with the immunochip platform in beh\u00e7et disease. Identification of residues associated in the HLA class I region and new susceptibility Loci","volume":"11","author":"Ortiz-Fern\u00e1ndez","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.phrs.2021.105593_bib20","doi-asserted-by":"crossref","first-page":"695","DOI":"10.3899\/jrheum.140949","article-title":"Variants of the IFI16 gene affecting the levels of expression of mRNA are associated with susceptibility to beh\u00e7et disease","volume":"42","author":"Ortiz-Fern\u00e1ndez","year":"2015","journal-title":"J. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib21","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2019.02755","article-title":"Association of functional polymorphisms of KIR3DL1\/DS1 with Beh\u00e7et\u2019s disease","volume":"10","author":"Casta\u00f1o-N\u00fa\u00f1ez","year":"2019","journal-title":"Front. Immunol."},{"key":"10.1016\/j.phrs.2021.105593_bib22","doi-asserted-by":"crossref","first-page":"1315","DOI":"10.1007\/s11033-012-2175-x","article-title":"Toll-like receptor polymorphisms and vasculitis susceptibility: meta-analysis and systematic review","volume":"40","author":"Song","year":"2013","journal-title":"Mol. Biol. Rep."},{"key":"10.1016\/j.phrs.2021.105593_bib23","doi-asserted-by":"crossref","first-page":"2141","DOI":"10.1093\/rheumatology\/kes238","article-title":"CC chemokine receptor 5 polymorphism in Italian patients with Behcet\u2019s disease","volume":"51","author":"Atzeni","year":"2012","journal-title":"Rheumatology"},{"key":"10.1016\/j.phrs.2021.105593_bib24","doi-asserted-by":"crossref","first-page":"8453","DOI":"10.1038\/s41598-017-09164-7","article-title":"Mutational profile of rare variants in inflammasome-related genes in Beh\u00e7et disease: a next generation sequencing approach","volume":"7","author":"Burillo-Sanz","year":"2017","journal-title":"Sci. Rep."},{"key":"10.1016\/j.phrs.2021.105593_bib25","doi-asserted-by":"crossref","first-page":"2777","DOI":"10.1038\/s41598-019-39113-5","article-title":"Beh\u00e7et\u2019s disease and genetic interactions between HLA-B*51 and variants in genes of autoinflammatory syndromes","volume":"9","author":"Burillo-Sanz","year":"2019","journal-title":"Sci. Rep."},{"key":"10.1016\/j.phrs.2021.105593_bib26","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1067\/mjd.2001.117728","article-title":"HSP 60 expression in mucocutaneous lesions of Beh?et\u2019s disease","volume":"45","author":"Ergun","year":"2001","journal-title":"J. Am. Acad. Dermatol."},{"key":"10.1016\/j.phrs.2021.105593_bib27","first-page":"S44","article-title":"The role of heat shock proteins in Beh\u00e7et\u2019s disease","volume":"21","author":"Direskeneli","year":"2003","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib28","first-page":"S96","article-title":"Serum levels of free heat shock protein 70 and anti-HSP70 are elevated in Beh\u00e7et\u2019s disease","volume":"26","author":"Birtas-Atesoglu","year":"2008","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib29","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1007\/s11739-013-1036-5","article-title":"Beh\u00e7et\u2019s syndrome pathophysiology and potential therapeutic targets","volume":"9","author":"Emmi","year":"2014","journal-title":"Intern. Emerg. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib30","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1080\/03009740600905380","article-title":"Expression of cytokines, chemokines, and chemokine receptors in oral ulcers of patients with Behcet\u2019s disease (BD) and recurrent aphthous stomatitis is Th1\u2010associated, although Th2\u2010association is also observed in patients with BD","volume":"35","author":"Dalghous","year":"2006","journal-title":"Scand. J. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib31","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1016\/0008-8749(92)90207-6","article-title":"Streptococcal-related antigens stimulate production of IL6 and interferon-\u03b3 by T cells from patients with Behcet\u2019s disease","volume":"140","author":"Hirohata","year":"1992","journal-title":"Cell. Immunol."},{"key":"10.1016\/j.phrs.2021.105593_bib32","doi-asserted-by":"crossref","first-page":"2001","DOI":"10.1002\/art.34327","article-title":"Interleukin-21 modulates Th1 and Th17 responses in giant cell arteritis","volume":"64","author":"Terrier","year":"2012","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.phrs.2021.105593_bib33","first-page":"S71","article-title":"Th17 cells in Beh\u00e7et\u2019s disease: a new immunoregulatory axis","volume":"29","author":"Hamzaoui","year":"2011","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib34","first-page":"2797","article-title":"Up-regulation of th17 and related cytokines in beh\u00e7et\u2019s disease corresponding to disease activity","volume":"31","author":"Na","year":"2013","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib35","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/gene.2014.64","article-title":"Activation of the JAK\/STAT pathway in Behcet\u2019s disease","volume":"16","author":"Tulunay","year":"2015","journal-title":"Genes Immun."},{"issue":"10","key":"10.1016\/j.phrs.2021.105593_bib36","doi-asserted-by":"crossref","first-page":"2797","DOI":"10.1007\/s10067-018-4049-7","article-title":"Correlation between IL-17A\/F, IL-23, IL-35 and IL-12\/-23 (p40) levels in peripheral blood lymphocyte cultures and disease activity in Behcet\u2019s patients","volume":"37","author":"Sonmez","year":"2018","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib37","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1111\/j.1365-2249.2011.04543.x","article-title":"Excessive CD4+ T cells co-expressing interleukin-17 and interferon-\u03b3 in patients with Beh\u00e7et\u2019s disease","volume":"168","author":"Shimizu","year":"2012","journal-title":"Clin. Exp. Immunol."},{"key":"10.1016\/j.phrs.2021.105593_bib38","first-page":"S59","article-title":"Preferential activation of circulating CD8+ and gammadelta T cells in patients with active Beh\u00e7et\u2019s disease and HLA-B51","volume":"26","author":"Yasuoka","year":"1991","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib39","first-page":"249","article-title":"IL-8 producing cells in patients with Beh\u00e7et\u2019s disease","volume":"18","author":"Mantas","year":"2000","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib40","doi-asserted-by":"crossref","first-page":"7678","DOI":"10.4049\/jimmunol.175.11.7678","article-title":"T Cell-regulated neutrophilic inflammation in autoinflammatory diseases","volume":"175","author":"Keller","year":"2005","journal-title":"J. Immunol."},{"key":"10.1016\/j.phrs.2021.105593_bib41","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.clim.2014.01.009","article-title":"Secretion of interleukin-17 by CD8+ T cells expressing CD146 (MCAM)","volume":"152","author":"Dagur","year":"2014","journal-title":"Clin. Immunol."},{"key":"10.1016\/j.phrs.2021.105593_bib42","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1136\/annrheumdis-2019-215356","article-title":"The role of IL-17A in axial spondyloarthritis and psoriatic arthritis: recent advances and controversies","volume":"78","author":"McGonagle","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib43","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1111\/j.1601-0825.2005.01185.x","article-title":"Diversity of gammadelta T cells in patients with Behcet\u2019s disease is indicative of polyclonal activation","volume":"12","author":"Freysdottir","year":"2006","journal-title":"Oral. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib44","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1007\/0-306-48382-3_53","article-title":"T-\u03b3\u03b4 receptor restriction in peripheral lymphocytes of patients with Beh\u00e7et\u2019s disease","author":"Van Hagen","year":"2004","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.phrs.2021.105593_bib45","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/j.humimm.2015.10.001","article-title":"In vitro evaluation of \u03b3\u03b4 T cells regulatory function in Beh\u00e7et\u2019s disease patients and healthy controls","volume":"77","author":"Clemente Ximenis","year":"2016","journal-title":"Hum. Immunol."},{"key":"10.1016\/j.phrs.2021.105593_bib46","doi-asserted-by":"crossref","first-page":"R15","DOI":"10.1186\/ar4147","article-title":"Expression of regulatory receptors on \u03b3\u03b4 T cells and their cytokine production in Behcet\u2019s disease","volume":"15","author":"Parlakgul","year":"2013","journal-title":"Arthritis Res. Ther."},{"key":"10.1016\/j.phrs.2021.105593_bib47","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2019.00665","article-title":"Immunopathogenesis of Behcet\u2019s disease","volume":"10","author":"Tong","year":"2019","journal-title":"Front. Immunol."},{"key":"10.1016\/j.phrs.2021.105593_bib48","doi-asserted-by":"crossref","first-page":"117","DOI":"10.2174\/1568010053622984","article-title":"Pharmacological and clinical basis of treatment of familial mediterranean fever (FMF) with colchicine or analogues: an update","volume":"4","author":"Cerquaglia","year":"2005","journal-title":"Curr. Drug Target Inflamm. Allergy"},{"key":"10.1016\/j.phrs.2021.105593_bib49","doi-asserted-by":"crossref","first-page":"1987","DOI":"10.1001\/archinte.165.17.1987","article-title":"Colchicine as first-choice therapy for recurrent pericarditis","volume":"165","author":"Imazio","year":"2005","journal-title":"Arch. Intern. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib50","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/j.semarthrit.2015.06.013","article-title":"Colchicine\u2014update on mechanisms of action and therapeutic uses","volume":"45","author":"Leung","year":"2015","journal-title":"Semin. Arthritis Rheum."},{"key":"10.1016\/j.phrs.2021.105593_bib51","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1172\/JCI118147","article-title":"Colchicine alters the quantitative and qualitative display of selectins on endothelial cells and neutrophils","volume":"96","author":"Cronstein","year":"1995","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.phrs.2021.105593_bib52","article-title":"A double blind study of colchicine in Behcet\u2019s disease","author":"Aktulga","year":"1980","journal-title":"Haematologica"},{"key":"10.1016\/j.phrs.2021.105593_bib53","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/S0140-6736(89)92381-7","article-title":"Double-masked trial of cyclosporin versus colchicine and long-term open study of cyclosporin in beh\u00e7et\u2019s disease","volume":"333","author":"Masuda","year":"1989","journal-title":"Lancet"},{"key":"10.1016\/j.phrs.2021.105593_bib54","doi-asserted-by":"crossref","DOI":"10.1002\/1529-0131(200111)44:11<2686::AID-ART448>3.0.CO;2-H","article-title":"A double-blind trial of colchicine in Beh\u00e7et\u2019s syndrome","author":"Yurdakul","year":"2001","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.phrs.2021.105593_bib55","doi-asserted-by":"crossref","first-page":"542","DOI":"10.3109\/s10165-009-0200-2","article-title":"Colchicine versus placebo in Beh\u00e7et\u2019s disease: randomized, double-blind, controlled crossover trial","volume":"19","author":"Davatchi","year":"2009","journal-title":"Mod. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib56","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2018-213225","article-title":"2018 update of the EULAR recommendations for the management of Beh\u00e7et\u2019s syndrome","author":"Hatemi","year":"2018","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib57","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1056\/NEJM199002013220501","article-title":"A controlled trial of azathioprine in Beh\u00e7et\u2019s syndrome","volume":"322","author":"Yazici","year":"1990","journal-title":"New Engl. J. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib58","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1002\/art.1780400425","article-title":"Azathioprine in Beh\u00e7et\u2019s syndrome. Effects on long-term prognosis","volume":"40","author":"Hamuryudan","year":"1997","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.phrs.2021.105593_bib59","doi-asserted-by":"crossref","first-page":"1733","DOI":"10.1002\/acr.20308","article-title":"Azathioprine in severe uveitis of Beh\u00e7et\u2019s disease","volume":"62","author":"Saadoun","year":"2010","journal-title":"Arthritis Care Res."},{"key":"10.1016\/j.phrs.2021.105593_bib60","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.rdc.2013.02.010","article-title":"Beh\u00e7et\u2019s syndrome","volume":"39","author":"Hatemi","year":"2013","journal-title":"Rheum. Dis. Clin. North Am."},{"key":"10.1016\/j.phrs.2021.105593_bib61","doi-asserted-by":"crossref","DOI":"10.1159\/000051781","article-title":"Mycophenolate mofetil is ineffective in the treatment of mucocutaneous Adamantiades-Beh\u00e7et\u2019s disease","author":"Adler","year":"2001","journal-title":"Dermatology"},{"key":"10.1016\/j.phrs.2021.105593_bib62","article-title":"Mycophenolate mofetil as a novel immunosuppressant in the treatment of neuro-Beh\u00e7et\u2019s disease with parenchymal involvement: presentation of four cases","author":"Shugaiv","year":"2011","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib63","article-title":"Evaluation of conventional therapy versus cyclosporine A in Behcet\u2019s syndrome","author":"BenEzra","year":"1988","journal-title":"Transplant. Proc."},{"key":"10.1016\/j.phrs.2021.105593_bib64","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1136\/bjo.76.4.241","article-title":"Low dose cyclosporin A versus pulsed cyclophosphamide in Behcet\u2019s syndrome: a single masked trial.","volume":"76","author":"Ozyazgan","year":"1992","journal-title":"Br. J. Ophthalmol."},{"key":"10.1016\/j.phrs.2021.105593_bib65","article-title":"Cyclosporine for Beh\u00e7et\u2019s uveitis: is it associated with an increased risk of neurological involvement?","author":"Akmar-Demir","year":"2008","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib66","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1007\/s10067-005-0070-8","article-title":"CNS involvement occurs more frequently in patients with Beh\u00e7et\u2019s disease under cyclosporin A (CSA) than under other medications\u2014results of a retrospective analysis of 117 cases","volume":"25","author":"K\u00f6tter","year":"2006","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib67","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1093\/rheumatology\/33.1.48","article-title":"Pulmonary arterial aneurysms in beh\u00e7et\u2019s syndrome: a report of 24 cases","volume":"33","author":"Hamuryudan","year":"1994","journal-title":"Rheumatology"},{"key":"10.1016\/j.phrs.2021.105593_bib68","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1016\/j.amjmed.2004.05.027","article-title":"Pulmonary artery aneurysms in Beh\u00e7et syndrome","volume":"117","author":"Hamuryudan","year":"2004","journal-title":"Am. J. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib69","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1016\/j.neurol.2011.09.006","article-title":"Neurological manifestations of Beh\u00e7et\u2019s disease: evaluation of 40 patients treated by cyclophosphamide","volume":"168","author":"Ait Ben Haddou","year":"2012","journal-title":"Rev. Neurol."},{"key":"10.1016\/j.phrs.2021.105593_bib70","doi-asserted-by":"crossref","first-page":"3072","DOI":"10.3390\/ijms21093072","article-title":"Beh\u04abet\u2019s disease, and the role of TNF-\u03b1 and TNF-\u03b1 blockers","volume":"21","author":"van der Houwen","year":"2020","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.phrs.2021.105593_bib71","article-title":"Short-term trial of etanercept in Beh\u00e7et\u2019s disease: a double blind, placebo controlled study","author":"Melikoglu","year":"2005","journal-title":"J. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib72","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1186\/s13075-019-1933-8","article-title":"Comparative study of corticosteroid monotherapy, and TNF inhibitors with or without corticosteroid in patients with refractory entero-Behcet\u2019s disease","volume":"21","author":"Miyagawa","year":"2019","journal-title":"Arthritis Res. Ther."},{"key":"10.1016\/j.phrs.2021.105593_bib73","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1007\/s10067-008-0857-5","article-title":"Efficacy of etanercept in the treatment of a patient with Beh\u00e7et\u2019s disease","volume":"27","author":"Curigliano","year":"2008","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib74","doi-asserted-by":"crossref","first-page":"51ii","DOI":"10.1136\/ard.61.suppl_2.ii51","article-title":"Behcet\u2019s disease: a new target for anti-tumour necrosis factor treatment","volume":"61","author":"Sfikakis","year":"2002","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib75","article-title":"Multicenter study of infliximab for refractory uveoretinitis in Beh\u00e7et disease","author":"Okada","year":"2012","journal-title":"Arch. Ophthalmol."},{"key":"10.1016\/j.phrs.2021.105593_bib76","article-title":"Long-term remission of ocular and extraocular manifestations in Beh\u00e7et\u2019s disease using infliximab","author":"Handa","year":"2011","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib77","article-title":"Efficacy of infliximab in refractory Beh\u00e7et\u2019s disease-associated and idiopathic posterior segment uveitis: a prospective, follow-up study of 50 patients","volume":"5","author":"Cantini","year":"2011","journal-title":"Biol. Targets Ther."},{"key":"10.1016\/j.phrs.2021.105593_bib78","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1136\/bjo.2010.194464","article-title":"Decreased ocular inflammatory attacks and background retinal and disc vascular leakage in patients with Behcet\u2019s disease on infliximab therapy","volume":"95","author":"Keino","year":"2011","journal-title":"Br. J. Ophthalmol."},{"key":"10.1016\/j.phrs.2021.105593_bib79","doi-asserted-by":"crossref","first-page":"198","DOI":"10.3109\/09273948.2012.670360","article-title":"Long-term efficacy and safety of infliximab in the treatment of Beh\u00e7et\u2019s disease","volume":"20","author":"Capella","year":"2012","journal-title":"Ocul. Immunol. Inflamm."},{"key":"10.1016\/j.phrs.2021.105593_bib80","doi-asserted-by":"crossref","first-page":"1644","DOI":"10.1016\/S0140-6736(01)06677-6","article-title":"Effect of infliximab on threatening panuveitis in Beh\u00e7et\u2019s disease","volume":"358","author":"Mu\u00f1oz-Fern\u00e1ndez","year":"2001","journal-title":"Lancet"},{"key":"10.1016\/j.phrs.2021.105593_bib81","doi-asserted-by":"crossref","first-page":"841","DOI":"10.1038\/sj.eye.6701689","article-title":"Long-term treatment of refractory posterior uveitis with anti-TNF\u03b1 (infliximab)","volume":"19","author":"Benitez-del-Castillo","year":"2005","journal-title":"Eye"},{"key":"10.1016\/j.phrs.2021.105593_bib82","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.semarthrit.2010.09.002","article-title":"Anti-TNF agents for beh\u00e7et\u2019s disease: analysis of published data on 369 patients","volume":"41","author":"Arida","year":"2011","journal-title":"Semin. Arthritis Rheum."},{"key":"10.1016\/j.phrs.2021.105593_bib83","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1186\/s13075-018-1793-7","article-title":"Safety and efficacy of infliximab in the treatment of refractory uveoretinitis in Beh\u00e7et\u2019s disease: a large-scale, long-term postmarketing surveillance in Japan","volume":"21","author":"Ohno","year":"2019","journal-title":"Arthritis Res. Ther."},{"key":"10.1016\/j.phrs.2021.105593_bib84","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1111\/1756-185X.13778","article-title":"Efficacy of TNF\u03b1 inhibitors for refractory vascular Beh\u00e7et\u2019s disease: a multicenter observational study of 27 patients and a review of the literature","volume":"23","author":"Aksoy","year":"2020","journal-title":"Int. J. Rheum. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib85","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1002\/acr.21557","article-title":"Beh\u00e7et\u2019s disease: successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis","volume":"64","author":"Adler","year":"2012","journal-title":"Arthritis Care Res."},{"key":"10.1016\/j.phrs.2021.105593_bib86","doi-asserted-by":"crossref","first-page":"184","DOI":"10.3109\/s10165-010-0370-y","article-title":"Efficacy of combination therapy of anti-TNF-\u03b1 antibody infliximab and methotrexate in refractory entero-Beh\u00e7et\u2019s disease","volume":"21","author":"Iwata","year":"2011","journal-title":"Mod. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib87","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.jns.2008.05.002","article-title":"Effect of infliximab in progressive Neuro-Beh\u00e7et\u2019s syndrome","volume":"272","author":"Kikuchi","year":"2008","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.phrs.2021.105593_bib88","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1002\/art.23345","article-title":"Infliximab for the treatment of Neuro-Beh\u00e7et\u2019s disease: a case series and review of the literature","volume":"59","author":"Pipitone","year":"2008","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.phrs.2021.105593_bib89","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/s00296-009-1213-z","article-title":"One year study of efficacy and safety of infliximab in the treatment of patients with ocular and neurological Beh\u00e7et\u2019s disease refractory to standard immunosuppressive drugs","volume":"31","author":"Giardina","year":"2011","journal-title":"Rheumatol. Int."},{"key":"10.1016\/j.phrs.2021.105593_bib90","first-page":"185","article-title":"Combination therapy with infliximab and methotrexate in recalcitrant mucocutaneous Beh\u00e7et disease","volume":"89","author":"Chan","year":"2012","journal-title":"Cutis"},{"key":"10.1016\/j.phrs.2021.105593_bib91","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1007\/s00296-009-1276-x","article-title":"Late response to anti-TNF-\u03b1 therapy in refractory mucocutaneous lesions of Beh\u00e7et\u2019s disease","volume":"31","author":"Aikawa","year":"2011","journal-title":"Rheumatol. Int."},{"key":"10.1016\/j.phrs.2021.105593_bib92","doi-asserted-by":"crossref","first-page":"1500","DOI":"10.1002\/art.40531","article-title":"Adalimumab-based treatment versus disease-modifying antirheumatic drugs for venous thrombosis in Beh\u00e7et\u2019s syndrome","volume":"70","author":"Emmi","year":"2018","journal-title":"Arthritis Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib93","doi-asserted-by":"crossref","DOI":"10.1007\/s10067-009-1272-2","article-title":"Adalimumab treatment for life threatening pulmonary artery aneurysm in Beh\u00e7et disease: a case report","author":"Lee","year":"2010","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib94","doi-asserted-by":"crossref","DOI":"10.1016\/j.crohns.2011.03.006","article-title":"Successful treatment with adalimumab in a familial case of gastrointestinal Behcet\u2019s disease","author":"De Cassan","year":"2011","journal-title":"J. Crohn\u2019s Colitis"},{"key":"10.1016\/j.phrs.2021.105593_bib95","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/kes130","article-title":"Adalimumab for the treatment of beh\u00e7et\u2019s disease: experience in 19 patients","author":"Perra","year":"2012","journal-title":"Rheumatology"},{"key":"10.1016\/j.phrs.2021.105593_bib96","article-title":"Efficacy of adalimumab in patients with Beh\u00e7et\u2019s disease unsuccessfully treated with infliximab","author":"Olivieri","year":"2011","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib97","article-title":"Successful treatment of leg ulcers in Beh\u00e7et\u2019s disease using adalimumab plus methotrexate after the failure of infliximab","author":"Atzeni","year":"2010","journal-title":"Clin. Exp. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib98","doi-asserted-by":"crossref","DOI":"10.3109\/09273948.2010.483314","article-title":"Clinical experience with adalimumab in the treatment of ocular Beh\u00e7et disease","author":"Bawazeer","year":"2010","journal-title":"Ocul. Immunol. Inflamm."},{"key":"10.1016\/j.phrs.2021.105593_bib99","doi-asserted-by":"crossref","first-page":"2081","DOI":"10.1002\/art.41026","article-title":"Comparative study of infliximab versus adalimumab in refractory uveitis due to beh\u00e7et\u2019s disease: national multicenter study of 177 cases","volume":"71","author":"Atienza\u2010Mateo","year":"2019","journal-title":"Arthritis Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib100","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1007\/s10067-018-4228-6","article-title":"Comparative efficacy between adalimumab and infliximab in the treatment of non-infectious intermediate uveitis, posterior uveitis, and panuveitis: a retrospective observational study of 107 patients","volume":"38","author":"Fabiani","year":"2019","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib101","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1016\/j.semarthrit.2020.05.017","article-title":"Effectiveness of tocilizumab in Behcet\u2019s disease: a systematic literature review","volume":"50","author":"Akiyama","year":"2020","journal-title":"Semin. Arthritis Rheum."},{"key":"10.1016\/j.phrs.2021.105593_bib102","doi-asserted-by":"crossref","first-page":"1727","DOI":"10.1002\/art.40912","article-title":"Long\u2010term outcome of ustekinumab therapy for Beh\u00e7et\u2019s disease","volume":"71","author":"Mirouse","year":"2019","journal-title":"Arthritis Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib103","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.jaut.2018.09.002","article-title":"Efficacy of the anti-IL 17 secukinumab in refractory Beh\u00e7et\u2019s syndrome: a preliminary study","volume":"97","author":"Di Scala","year":"2019","journal-title":"J. Autoimmun."},{"key":"10.1016\/j.phrs.2021.105593_bib104","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1136\/annrheumdis-2020-217108","article-title":"Long-term effectiveness and safety of secukinumab for treatment of refractory mucosal and articular Beh\u00e7et\u2019s phenotype: a multicentre study","volume":"79","author":"Fagni","year":"2020","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib105","doi-asserted-by":"crossref","first-page":"1510","DOI":"10.1056\/NEJMoa1408684","article-title":"Apremilast for Beh\u00e7et\u2019s syndrome \u2014 a phase 2, placebo-controlled study","volume":"372","author":"Hatemi","year":"2015","journal-title":"New Engl. J. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib106","doi-asserted-by":"crossref","first-page":"1918","DOI":"10.1056\/NEJMoa1816594","article-title":"Trial of apremilast for oral ulcers in Beh\u00e7et\u2019s syndrome","volume":"381","author":"Hatemi","year":"2019","journal-title":"New Engl. J. Med."},{"key":"10.1016\/j.phrs.2021.105593_bib107","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1136\/annrheumdis-2019-eular.1651","article-title":"Efficacy of apremilast for oral ulcers associated with active beh\u00e7et\u2019s syndrome over 64 weeks: results from a phase iii study","volume":"78","author":"Hatemi","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.phrs.2021.105593_bib108","article-title":"Efficacy of apremilast for oral ulcers associated with active Beh\u00e7et\u2019s syndrome over 64 weeks: long-term results from the japanese subgroup in a phase iii study","volume":"71","author":"Takeno","year":"2019","journal-title":"Arthritis Rheumatol. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib109","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1093\/rheumatology\/kez267","article-title":"Efficacy and safety of apremilast for Beh\u00e7et\u2019s syndrome: a real-life single-centre Italian experience","volume":"59","author":"De Luca","year":"2020","journal-title":"Rheumatology"},{"key":"10.1016\/j.phrs.2021.105593_bib110","series-title":"SAT0215 apremilast in refractory oral and\/or genital ulcers in beh\u00e7et\u2019s disease. multicenter study of 49 cases in clinical practice","first-page":"1182.2","author":"Atienza-Mateo","year":"2019"},{"key":"10.1016\/j.phrs.2021.105593_bib111","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1080\/14397595.2019.1696504","article-title":"Positioning of apremilast in treatment of Beh\u00e7et\u2019s disease","volume":"30","author":"Takeno","year":"2020","journal-title":"Mod. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib112","first-page":"4246965","article-title":"Gene expression profiling in Behcet\u2019s disease indicates an autoimmune component in the pathogenesis of the disease and opens new avenues for targeted therapy","volume":"2018","author":"Puccetti","year":"2018","journal-title":"Immunol. Res. Actions"},{"key":"10.1016\/j.phrs.2021.105593_bib113","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1097\/ICO.0000000000000019","article-title":"Effects of topical janus kinase inhibition on ocular surface inflammation and immunity","volume":"33","author":"Stevenson","year":"2014","journal-title":"Cornea"},{"key":"10.1016\/j.phrs.2021.105593_bib114","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.ajoc.2018.12.001","article-title":"Tofacitinib for refractory uveitis and scleritis","volume":"13","author":"Paley","year":"2019","journal-title":"Am. J. Ophthalmol. Case Rep."},{"key":"10.1016\/j.phrs.2021.105593_bib115","doi-asserted-by":"crossref","first-page":"1232","DOI":"10.1080\/09273948.2019.1605453","article-title":"Effect of janus kinase inhibitor treatment on anterior uveitis and associated macular edema in an adult patient with juvenile idiopathic arthritis","volume":"27","author":"Bauermann","year":"2019","journal-title":"Ocul. Immunol. Inflamm."},{"key":"10.1016\/j.phrs.2021.105593_bib116","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1007\/s10067-019-04875-w","article-title":"JAK inhibitors in refractory juvenile idiopathic arthritis-associated uveitis","volume":"38","author":"Miserocchi","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"10.1016\/j.phrs.2021.105593_bib117","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.1136\/annrheumdis-2020-217307","article-title":"A pilot study of tofacitinib for refractory Behcet\u2019s syndrome","volume":"79","author":"Liu","year":"2020","journal-title":"Ann. Rheum. Dis."}],"container-title":["Pharmacological Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1043661821001778?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1043661821001778?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T22:28:09Z","timestamp":1760912889000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1043661821001778"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5]]},"references-count":117,"alternative-id":["S1043661821001778"],"URL":"https:\/\/doi.org\/10.1016\/j.phrs.2021.105593","relation":{},"ISSN":["1043-6618"],"issn-type":[{"value":"1043-6618","type":"print"}],"subject":[],"published":{"date-parts":[[2021,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Beh\u00e7et disease: From pathogenesis to novel therapeutic options","name":"articletitle","label":"Article Title"},{"value":"Pharmacological Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.phrs.2021.105593","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"105593"}}